Ragab Ahmed A Y, Houck Charlotte A, van der Does Lisette J M E, Lanters Eva A H, Burghouwt Danielle E, Muskens Agnes J Q M, de Groot Natasja M S
Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands.
Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands.
Am J Cardiol. 2017 Aug 1;120(3):428-434. doi: 10.1016/j.amjcard.2017.04.044. Epub 2017 May 11.
Brugada syndrome (BrS) is an autosomal dominant channelopathy which is responsible for a large number of sudden cardiac deaths in young subjects without structural abnormalities. The most challenging step in management of patients with BrS is identifying who is at risk for developing malignant ventricular tachyarrhythmia (VTA). In patients with BrS, conduction delay in the right ventricular outflow tract (RVOT) causes a prominent R wave in lead aVR. This electrocardiographic parameter can be useful to detect these high-risk patients. The goal of this study was to test the significance of R-wave elevation in lead aVR as a predictor for VTA in patients with BrS. In this retrospective study, we included 132 patients with BrS (47 ± 15 years, 65% men) who visited the outpatient clinic for cardiogenetic screening. Patients' medical records were examined for the presence of a positive R-wave sign in lead aVR and VTA. A positive R-wave sign in lead aVR was observed in 41 patients (31%). This sign was more frequently observed in patients who experienced VTA (n = 24) before the initial diagnosis, during electrophysiological studies, or during follow-up (p <0.001). The positive R-wave sign occurred more frequently in symptomatic patients with a history of an out of hospital cardiac arrest, VTA, or syncope than asymptomatic patients (60% vs 26%; p = 0.002). During the follow-up period, this sign was more frequently detected in patients who developed either de novo (50%) or recurrent VTA (80%) (p = 0.017). Multivariable regression analysis showed that R-wave sign is an independent predictor for VTA development (odds ratio 4.8, 95% confidence interval 1.79 to 13.27). The presence of a positive R-wave sign in lead aVR is associated with the development of VTA. In conclusion, positive R-wave sign in lead aVR can be used to identify patients with BrS at risk for malignant VTA.
布加综合征(BrS)是一种常染色体显性通道病,是导致大量无结构异常的年轻患者心源性猝死的原因。布加综合征患者管理中最具挑战性的步骤是确定谁有发生恶性室性心律失常(VTA)的风险。在布加综合征患者中,右心室流出道(RVOT)的传导延迟导致aVR导联出现明显的R波。这一心电图参数有助于检测这些高危患者。本研究的目的是检验aVR导联R波抬高作为布加综合征患者VTA预测指标的意义。在这项回顾性研究中,我们纳入了132例因心脏遗传学筛查前来门诊就诊的布加综合征患者(47±15岁,65%为男性)。检查患者病历中是否存在aVR导联R波阳性体征及VTA情况。41例患者(31%)观察到aVR导联R波阳性体征。该体征在初始诊断前、电生理检查期间或随访期间发生VTA的患者中更常见(n = 24)(p<0.001)。有院外心脏骤停、VTA或晕厥病史的有症状患者中,R波阳性体征的出现频率高于无症状患者(60%对26%;p = 0.002)。在随访期间,新发(50%)或复发VTA(80%)的患者中更频繁检测到该体征(p = 0.017)。多变量回归分析显示,R波体征是VTA发生的独立预测指标(比值比4.8,95%置信区间1.79至13.27)。aVR导联R波阳性体征的存在与VTA的发生相关。总之,aVR导联R波阳性体征可用于识别有恶性VTA风险的布加综合征患者。